Cystic Fibrosis

CF is a serious life-long condition, but the severity of the illness can vary greatly. The average life expectancy for someone with CF is about 35 years. Although, some with mild forms of CF can live to age 60 or beyond.

Causes

CF is a genetic disorder. A child with CF inherits defective genes from each parent. Parents who have the gene, but do not have CF, are called carriers.

Risk Factors

Factors that increase your chance of CF include:

Parents who are known carriers of the CF gene

Siblings with CF

Parents with CF—mostly the mother since men with CF are often sterile

Symptoms

The abnormally thick mucus of CF blocks certain organs. This causes many of the symptoms of CF.

Symptoms in infants may include:

Difficulty passing the first stool

Salty sweat

Intestinal obstruction, sometimes requiring surgery

Mucus that causes blockages in the lungs may lead to:

Coughing and wheezing

Shortness of breath

Difficulty with exercise

Abnormally shaped clubbed finger tips

Malformed chest

Mucus can also block the pancreas. This can block enzymes used to help you digest food. This can lead to:

Diagnosis

The doctor will ask about symptoms and medical history. A physical exam will be done. CF is suspected in a child with classic symptoms, especially if a sibling has CF.

CF is often diagnosed by symptoms, family history of CF, or a positive screening test in newborns. The diagnosis may be confirmed with genetic testing. Other lab tests that may be used to confirm CF include:

Sweat chloride testing

Transepithelial nasal potential difference measurement

Your doctor may need to check your lungs. This may be done to look for symptoms or to determine treatment. Tests may include:

Tests may also be needed to check the pancreas. These tests may be done to assess symptoms or determine treatment.

Treatment

There is no cure for CF. Treatment is aimed at:

Improving the amount of nutrition your body receives

Preventing and treating lung and sinus infections

Keeping the airways and lungs as clear as possible

Treatment for CF includes:

Nutritional Support

Better nutrition will help improve overall health. It will also improve growth and development in children. Children who have returned to normal weight within 2 years of the diagnosis have fewer coughing episodes and better lung function. Some nutritional steps that may help include:

A high-calorie diet planned by a registered dietitian

Nutritional supplements, including fat-soluble vitamins

Pancreatic enzyme tablets with meals to improve digestion and absorption of nutrients

Drinking lots of fluids and salt replacement, especially in hot weather or during illnesses

Managing Lung Infections

Thick mucus in the airways increases the risk of respiratory infections. The infection can also be more severe because of the mucus. Treatment of a current infection often requires antibiotics. Prevention of new infections may be done with:

Vaccination

Antibiotics (usually inhaled)

Lung and Airway Support

Medications help keep the airways clear. Most will be delivered through an inhaler or nebulizer. Medications may include:

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